Not quite one in a million

Around the same time as Cat died I was diagnosed with a rare adrenal gland tumour called a pheochromocytoma. I say rare but according to Wikipedia there are approximately two to eight per million. Does that make it rare? Wikipedia goes on to say that when they were mentioned in ER they “usually generated interest from the doctors due to the rarity of the diagnosis.” That fits with my experience too actually as the various doctors I have seen have been quite fascinated by my case and several have mentioned me being  a case study they may write up.

Let’s take a figure in the middle of the quoted range and say there are five cases per million. In the UK that means there are something like 300 pheochromocytomas, right? They are apparently more prevalent in young or middle aged adults so we could potentially adjust that figure down to exclude the very young and the elderly. Say something like 200 cases then. Say 150 if you prefer. Whatever. The fact remains that there are more people in the UK with this rare tumour than people currently managing a Football League club. Every club has one and I’m sure you could name several but would you class football managers as rare?

Interestingly the doctors I have seen have been referring to my “pheo” or calling it a “mass” or occasionally a “lump”. Is this a sign of the times, dressing things up so as to avoid offence? Can the NHS not call a spade a spade? Have some focus group decreed that the word tumour has too many negative connotations and has been show to unduly worry patients so staff have to use the term “mass” instead? I prefer to call it what it is – a tumour.

If there has been a memo sent round the NHS asking staff to use “mass” rather than tumour it obviously hasn’t reached everyone. My mum (who is an oncology nurse or something) was talking to someone at her hospital a while ago about my condition. On hearing what I had the fella said something along the lines of “Pheochromocytoma? Isn’t a malignancy of the adrenal gland?” Exactly. There’s a damn good chance my tumour is benign but at least calling it a malignancy gets the message across better than “mass” does. Mass just makes it sound like a harmless bit of extra tissue, not the root cause of my health problems for the last two years!

From what I understand this pheo has been at the root of everything wrong with me. I first reported abdominal pains to my GP in January 2009 and eventually got referred to the gastroenterology clinic at the hospital. They spent a year – on and off – doing various tests all of which came back normal. During one of the early tests a student nurse spotted my blood pressure was high. Hypertension runs in the family somewhat so after a quick visit to my GP I was given drugs to control that. Meanwhile I was being fobbed off with a diagnosis of IBS. I spent several sessions with a psychologist in case the problem had a mental cause and after some CBT (cognitive behavioural therapy, not cock and ball torture) I was pain-free. Yes! Subconscious stress seems to have been the main issue.

Around the same time the gastroenterology clinic were having one last throw of the dice – a 24 hour urine test. Aha! It may not be anything to worry about but my noradrenaline level was way off the scale. I was discharged from that clinic and referred to endocrinology in case this noradrenaline level was of concern to them. It was. Very much so. They repeated the test another couple of times to be sure and in doing so confirmed their suspicions that I had a tumour on one of my adrenal glands. In fact they went back over my CT scans from some months before (ignoring the original report that said my adrenals were normal, incidentally) and picked out the lump quite nicely. Apparently. I haven’t seen the pictures, unfortunately. Abdominal pain and hypertension – classic signs of a pheo. But who was going to stick their neck out and diagnose that without plenty of evidence to back their diagnosis up?

So there we have it, a lump. And what do we do with a lump? We cut it out. As well as the lump, the adrenal gland itself has to come out too. But it’s not so bad as the other one just takes up the slack apparently. But before the surgeon can sharpen his knife I needed to be given a load of drugs. I was likened to a ticking timebomb by the endocrinologist and it was his job to chemically dampen me before the surgeon effectively defused me. The first stage was to get me alpha blocked, then he could add beta blockers to the mix and once they kicked in I was good to go. At this stage I was introduced to what has been the bane of the last few months for me: phenoxybenzamine.

Alpha blockers relax certain muscles and help blood vessels stay open, rather than being closed up by the action of noradrenaline and thus lower blood pressure. I was to come off the blood pressure meds and take phenoxybenzamine instead. In doing so I went from feeling pretty good to feeling fucking awful! The patient information leaflet for phenoxybenzamine lists loads of side effects and I was steadily ticking them off. Nasal congestion – yes. Lightheadedness – yes. Dizziness – yes. Nausea – yes. Tiredness or feeling faint – yes. But my blood pressure was only slightly lower than it had been. It seemed as though the pheo was working flat out to counter the effect of the drugs – yay! So the phenoxybenzamine dose was steadily upped and with that the side effects worsened. Weak muscles – yes. Complete exhaustion – yes.

All the time Christmas was getting closer. Unless these drugs kicked in properly soon then there was no way the op would take place this side of Christmas, in which case I would be on these nasty drugs for even longer. It was mid-November before the hospital got the phenoxybenzamine dose right and could then add propranalol into the mix. Even then it could another couple of weeks to get that dose right too. But finally we were there. And by there I mean a place where the surgeon would be happy to even think about operating. Coincidentally this is the same place where a short walk to the shops and back leaves me knackered, I feel faint when I stand up, I often wake in the night with pins and needles in my limbs, I am constantly tired regardless of how much sleep I get and I am usually in bed by 9pm. My blood pressure is low, I have crippling abdominal pain at times and I generally feel like shit too. But at least the surgeon will operate now.

Well not now as such, but in a couple of days. Unless they cancel on me at the last minute (which I am told is possible) then I go under the knife on Wednesday. I’ll be in hospital for anything from a couple of days to a couple of weeks, depending. Ideally the surgery will be done laparoscopically (keyhole) in which case recovery times are much shorter but in some cases – especially those of larger tumours and mine is around 3cm x 2cm apparently, which makes it a similar sort of size to the adrenal gland I think – they need to really cut you open and then you can expect to be in hospital much longer. I won’t know whether my op was laparoscopic or not until I wake up in intensive care and look down to see how much of a mess my left-hand side is.

Still, once I have recovered after the op my abdominal pain should have gone, my blood pressure should be normal and I should be healthy once more. At least that’s the plan…

Anyway, it’s nearly three months that I have been on these drugs so three months of complete exhaustion and feeling really rough. That’s badly affected my reading. On the face of it I would have thought that being in bed by 9pm most nights would mean I had a lot more time to read but it’s not worked out that way at all. I have been so tired I have been reading less than normal. And reading less means a lack of compulsion to update this blog. See, I knew I could get this back on track even if it has taken me nearly 1500 words.

And I’m still not done with the excuses…